Fibrodysplasia Ossificans Treatment & Management

Updated: Jul 18, 2017
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

Fibrodysplasia ossificans progressiva (FOP) should be diagnosed during the neonatal period. [28] Early treatment of fibrodysplasia ossificans progressiva helps avoid the factors of aggravation, slow the progression of the disease, and provide the children with improved quality of life. No effective medical therapy is known for fibrodysplasia ossificans progressiva; bisphosphonates and corticosteroids are only beneficial during the flares. Gene therapy may hold promise in fibrodysplasia ossificans progressiva treatment.

Systemic steroids are sometimes used for acute flare-ups. Iontophoresis with steroids or acetic acid may improve diminished range of motion.

The discovery of the FOP gene reveals a highly conserved target in the transforming growth factor-beta/bone morphogenetic protein signaling pathway and compels therapeutic approaches for the development of small-molecule signal transduction inhibitors for activinlike kinase-2. [29] Effective therapies for fibrodysplasia ossificans progressiva may be based on blocking activinlike kinase-2 or blocking of activin receptor IA/activin–like kinase 2 signaling. [30]

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Surgical Care

Patients with fibrodysplasia ossificans, a rare disorder, may require oral surgical and anesthetic procedures to control oral pain. The importance of a minimally invasive surgical technique and appropriate anesthetic management has been stressed. [31]

The experience using general anesthesia has been favorable, with awake nasal fiberoptic intubation evaluated as desirable for airway management. [32]

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